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Chilblain Lupus Erythematosus

Chilblain lupus


Presentation

  • Symptoms and signs were present by 8 weeks of her first pregnancy and had resolved by 10 days after delivery. The condition is discussed with particular emphasis on the relationship with pregnancy.[ncbi.nlm.nih.gov]
  • Intense papillary dermal edema is present. A superficial and deep perivascular lymphocytic infiltrate is seen, with the described "fluffy edema" of vessel walls. Lymphocytic vasculitis may be present.[dermaamin.com]
  • This article reviews the clinical presentation, pathogenesis, diagnosis and treatment of CHLE. As an index patient with unique features, we report a 13-year-old boy developing CHLE after anorexia nervosa. Sequencing of TREX1 was normal.[ncbi.nlm.nih.gov]
  • Chilblain lupus erythematosus with depigmentation is a rare presentation of lupus erythematosus that may simulate vitiligo. A 52-year-old lady with such a manifestaiton is being reported.[ucdavis.pure.elsevier.com]
  • However, when patients present with isolated chilblain lesions, the diagnosis of LE may be difficult. We report a case of chilblain LE who presented with depigmented macules on the acral areas and face resembling acrofacial vitiligo.[ijdvl.com]
Cushingoid
  • Osteoporosis, myopathy and cushingoid changes are additional adverse reactions that can be avoided with short therapeutic courses. Of note, osteonecrosis can occur with even short courses of prednisone.[psychiatryadvisor.com]
Increased Susceptibility to Infections
  • Apart from arthralgias, there is no evidence for internal-organ disease or an increased susceptibility to infection.[edoc.mdc-berlin.de]
Abdominal Cramps
  • The most common adverse reaction from this therapeutic is GI side effects, including nausea, vomiting, diarrhea, and abdominal cramps. These symptoms are typically dose dependent and may be avoided by starting at a lower dose.[psychiatryadvisor.com]
Scaly Rash
  • On examination she had a purpuric scaly rash on the fingers of the right hand and livido reticularis was noted on her legs.[ncbi.nlm.nih.gov]
  • Discoid lupus (also known as chronic cutaneous lupus erythematosus) is generally milder than SLE and usually appears as a red scaly rash on sun exposed areas such as the face, scalp, arms, legs or trunk.[healthjade.com]
Yellow Discoloration of the Skin
  • It may cuase yellow discoloration of the skin. If the combination of hydroxychloroquine and quinacrine are ineffective, two options are (1) switch to chloroquine and quinacrine or (2) start dapsone 50mg daily.[psychiatryadvisor.com]
Hemorrhagic Bullae
  • To the Editor: A 20-year-old man with no notable medical history presented to our dermatology clinic for evaluation of mildly painful, hemorrhagic bullae on the bilateral halluces of 1 month’s duration.[mdedge.com]

Workup

  • Serologic workup revealed increased antinuclear antibody titers of 1:320 (reference range, reference range.[mdedge.com]
  • The presence of other signs and symptoms suggestive of a hematologic disorder should prompt a systemic workup.[derm101.com]
  • Clinicians can distinguish between these lesions through histologic analysis and workup for systemic lupus. 7 Lupus pernio occurs secondary to sarcoidosis and is characterized by violaceous nodules and plaques on the central face. 8 Cryoglobulemia and[podiatrytoday.com]
  • (See Workup and Treatment .) SCLE lesions heal without scarring or atrophy, but they may result in dyspigmentation, which can be prominent. Severe systemic disease is unusual, but when it occurs, the patient may develop life-altering sequelae.[emedicine.medscape.com]
  • PubMed View Article Google Scholar Swart JF, Wulffraat NM: Diagnostic workup for mixed connective tissue disease in childhood. Isr Med Assoc J 2008, 10: 650–2.[ped-rheum.biomedcentral.com]

Treatment

  • None of the usual treatments for cutaneous LE is effective for the chilblain lesions, probably because none is directed towards prevention and treatment of the microvascular stasis.[ncbi.nlm.nih.gov]
  • Discoid lupus erythematosus lesions respond more quickly to treatment than chilblain lupus erythematosus lesions. Treatment with antimalarial agents, prednisone, pentoxifylline, or dapsone was of benefit to our patients.[ncbi.nlm.nih.gov]
  • We report two patients with chilblain lupus erythematosus of Hutchinson (CL) who responded to surgical treatment. One of them was a 72-year-old woman (case 1), and the other a 62-year-old man (case 2).[ncbi.nlm.nih.gov]
  • In this paper we report a case of chilblain lupus erythematosus that was diagnosed using the Mayo Clinic Diagnostic Criteria and its successful treatment with hydroxychloroquine.[ncbi.nlm.nih.gov]
  • Treatments for chilblain lupus such as antimalarial agents, prednisone, pentoxifylline, nifedipine, and dapsone are reported to be beneficial. Natural treatments should also be considered in the treatment of chilblain lupus.[drgaryslupustreatment.org]

Prognosis

  • […] to create an awareness of this autoimmune disorder including how to investigate and manage it, and the possibility of underlying systemic lupus erythematosus or progression to develop systemic lupus erythematosus, which is known to have an improved prognosis[ncbi.nlm.nih.gov]
  • Prognosis Special patient groups[amboss.com]

Etiology

  • Apart from rare monogenic deficiencies of complement factors, where lupuslike disease may occur in association with other autoimmune diseases or high susceptibility to bacterial infections, its etiology is multifactorial in nature.[edoc.mdc-berlin.de]
  • Its etiology is unknown, but the high level of autoantibodies in persons with the condition indicates a defect in the regulatory mechanisms that sustain self-tolerance and prevent the body from attacking its own cells, cell constituents, and proteins.[medical-dictionary.thefreedictionary.com]
  • Figure 5: Etiology of primary and secondary perniosis.[skin-diseases-and-skin-care.imedpub.com]
  • Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women.[msdmanuals.com]

Epidemiology

  • Epidemiology Sex : (10:1) ; more than 90% of cases reported in women Peak incidence : women aged 20–40 years ; no particular age of manifestation in men US prevalence : highest in African-American, Hispanic, and Asian populations References: [1] Epidemiological[amboss.com]
  • Nonfreezing Cold-Induced Injuries in Auerbach's Wilderness Medicine, 2-Volume Set, 7th Edition Links: epidemiology risk factors clinical features differential diagnosis management chapping cold injuries non freezing cold injuries (NFCI)[gpnotebook.co.uk]
  • CLE EPIDEMIOLOGY • ACLE All races are affected much more common in women than men (8:1). the malar rash in 20-60% of patients in LE Age – the malar rash is associated with a younger age of disease onset 9.[slideshare.net]
  • Drug-induced pernio: Sulindac-induced cases have been reported. [12] Epidemiology Frequency United States The true incidence of pernio is unknown because pernio frequently is unrecognized or misdiagnosed.[emedicine.medscape.com]
  • Epidemiology  More common in African American’s and Puerto Rican populations  Female Male  Usually with long- standing systemic, usually pulmonary disease 6.[de.slideshare.net]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology of cutaneous lupus erythematosus . Clin Rev Allergy Immunol 2007; 2013 :85–106 [ PubMed ] [ Google Scholar ] 8. Weizel J, Brahler S, Baler R, et al.[ncbi.nlm.nih.gov]
  • Pathophysiology of cutaneous lupus erythematosus. Clin Rev Allergy Immunol 2007; 2013 :85–106 [ PubMed ] [ Google Scholar ] 8. Weizel J, Brahler S, Baler R, et al.[ncbi.nlm.nih.gov]
  • The pathophysiology of the lesion is still not clearly known.[ijdvl.com]

Prevention

  • None of the usual treatments for cutaneous LE is effective for the chilblain lesions, probably because none is directed towards prevention and treatment of the microvascular stasis.[ncbi.nlm.nih.gov]
  • Treatment of specific manifestations of SLE is aimed at prevention of complications.[medical-dictionary.thefreedictionary.com]
  • Medical Care Prophylactic warming of acral areas, achieved by heat and appropriate clothing, best prevents pernio.[dermaamin.com]
  • Prevention of chilblains To prevent chilblains: Avoid or limit your exposure to cold. Dress in layers of loose clothing and wear mittens and warm, water-resistant footwear.[healthjade.com]
  • Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive[jamanetwork.com]

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